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Updated: Jun 3 2022

Soft Tissue Sarcoma

4.1

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Images
https://upload.orthobullets.com/topic/8049/images/adductor sarcoma.jpg
https://upload.orthobullets.com/topic/8049/images/angiosarcoma.jpg
https://upload.orthobullets.com/topic/8049/images/Histology C - parsons_moved.jpg
https://upload.orthobullets.com/topic/8049/images/pleomorphic liposarcoma_moved.jpg
https://upload.orthobullets.com/topic/8049/images/51c_moved.jpg
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  • summary
    • Soft Tissue Sarcomas are rare, malignant soft tissue tumors comprising of a variety of subtypes distinguished by histological findings. 
    • The condition usually presents in patients > 15 years old with a slow-growing, painless soft tissue mass.
    • Diagnosis is made by biopsy and histological findings. 
    • Treatment usually involves mass excision and radiation therapy.
  • Epidemiology
    • Incidence
      • 7000 new case/year in the United States
    • Demographics
      • males > females
      • 85% occur in patients >15 years old
    • Anatomic location
      • 60% occur in extremities
  • Etiology
    • Genetics
      • classic translocations
        • t(X;18); SYT-SSX fusion
          • synovial sarcoma
        • t(2:13)
          • rhabdomyosarcoma (alveolar)
        • t(12;16)(q13:p11)
          • myxoid liposarcoma
    • Associated conditions
      • neurofibromatosis type-1
        • malignant peripheral nerve sheath tumor (MPNST)
      • Stuart-Treves syndrome
        • angiosarcoma
  • Classification
    • Classification of STS is based on histology, with over 50 types recognized
      • all STS have the same presentation, imaging, differential diagnosis, and treatment
      • histologic findings and molecular signatures are used to distinguish individual types
      • commonly tested soft tissue sarcomas include
        • undifferentiated pleomorphic sarcoma (UPS; previously malignant fibrous histiocytoma)
        • malignant peripheral nerve sheath tumor (MPNST)
        • synovial sarcoma
        • liposarcoma
        • rhabdomyosarcoma
        • fibrosarcoma
        • leiomyosarcoma
        • epithelioid sarcoma
        • angiosarcoma
        • dermatofibrosarcoma protuberans
        • clear cell sarcoma
        • alveolar soft part sarcoma (ASPS)
    • AJCC staging system is used for interdisciplinary communication
      • AJCC Staging System for Soft Tissue Sarcoma of the Extremity or Trunk
      • Stage
      • Size
      • Lymph Node Involvement
      • Metastasis
      • Grade
      • IA
      • < 5 cm
      • None/unknown
      • None
      • Low
      • IB
      • > 5 cm
      • None/unknown
      • None
      • Low
      • II
      • < 5 cm
      • None/unknown
      • None
      • Intermediate/high
      • IIIA
      • 5-10 cm
      • None/unknown
      • None
      • Intermediate/high
      • IIIB
      • > 10 cm
      • None/unknown
      • None
      • Intermediate/high
      • IVA
      • Any
      • Regional
      • None
      • Any
      • IVB
      • Any
      • Any
      • Present
      • Any
  • Presentation
    • History
      • may be a history of trauma that draws attention to the mass
    • Symptoms
      • slow growing, painless mass
        • incidental trauma often draws attention to mass
    • Physical exam
      • inspection
        • mass may be palpable
        • lack of ecchymosis suggests an encapsulated mass
          • could contradict diagnosis of hematoma, which typically presents with ecchymosis after trauma
      • palpable soft tissue lesion
        • differentiation of whether a mass is above or below fascia has prognostic importance
        • size > 5 cm in cross-section is a poor prognostic factor
      • can be confused with more common pathology
        • lipoma
        • hematoma
  • Imaging
    • Radiographs
      • indications
        • obtain plain radiographs in at least two planes for initial workup
    • CT chest
      • indications
        • obtain to assess for metastatic disease
    • MRI
      • indications
        • mandatory to evaluate soft tissue lesions in the extremities and determine treatment algorithm
      • findings
        • T1: low signal intensity (isointense with muscle)
        • T2: high signal intensity
        • IV gadolinium: peripheral enhancing zone and non-enhancing necrotic center
      • can be diagnostic for the following benign lesions
        • lipoma
        • neurilemoma (schwannoma)
        • intramuscular myxoma
        • if MRI is diagnostic and the mass is benign and symptomatic, then it can be removed without a biopsy
      • indeterminate MRI
        • if MRI is indeterminate or suggestive of sarcoma, then a core needle or open biopsy must be obtained before further treatment is initiated
        • soft tissue sarcomas can look similar to hematomas, so be cautious of a "hematoma" which occurs without trauma
  • Studies
    • Histology
      • determined by type of sarcoma
        • undifferentiated pleomorphic sarcoma
        • malignant peripheral nerve sheath tumor
        • synovial sarcoma
        • liposarcoma
        • rhabdomyosarcoma
        • fibrosarcoma
        • leiomyosarcoma
        • epithelioid sarcoma
        • angiosarcoma
        • dermatofibrosarcoma protuberans
  • Differential
    • Differentiate from benign soft tissue masses with MRI and biopsy
      • Hematoma
      • Lipoma
      • Intramuscular Myxoma
      • Hemangioma of Soft Tissue
      • Calcifying Aponeurotic Fibroma
      • Plantar Fibromatosis (Ledderhose Disease)
      • Extra-abdominal Desmoid Tumor
      • Nodular Fasciitis
      • Neurofibroma
      • Neurilemmoma
      • Neuroma
  • Treatment
    • Operative
      • biopsy
        • indications
          • mass concerning for malignancy after clinical and radiological workup
        • outcomes
          • best results if done at the institution where definitive surgery will take place, under a multidisciplinary team
      • wide surgical resection & radiation therapy
        • indications
          • standard of care in most cases
          • treatment must be based on a tissue diagnosis unless images are diagnostic (i.e. lipoma)
          • radiation is an important adjunct to surgery decreasing local recurrence
          • chemotherapy is controversial for soft tissue sarcomas
            • considered standard of care for rhabdomyosarcoma and Ewing sarcoma
        • outcomes
          • reduces risk of local recurrence to less than 10%
      • amputation
        • indications
          • when otherwise unable to resect tumor with negative margins
            • most frequently in the foot
          • resection would result in irreversible damage to major nerves
          • patient comorbidities limit potential for recovery after limb-sparing surgery
        • outcomes
          • good prognosis in absence of metastatic disease
      • surgical resection of lung metastases
        • indications
          • first-line for pulmonary metastases if the preop evaluation shows that complete resection is possible
        • outcomes
          • curative in up to 25% of patients
  • Techniques
    • Biopsy
      • approach
        • longitudinal incision
          • transverse incisions are contraindicated due to a need for wider resection at time of surgery
      • technique
        • use anatomic route that limits contamination of compartments and neurovascular structures
        • maintain meticulous hemostasis
        • use knife or curette to remove tissue
        • drains should be placed distal and in line with the surgical incision to allow removal of drain site with extensile incision
    • Wide resection & radiation therapy
      • approach
        • elliptical incision used to incorporate biopsy and drain sites
      • technique
        • resection
          • use of tourniquet without exsanguination
          • maintain meticulous hemostasis
          • meticulous dissection to ensure wide margins, must confirm border free of disease with histology
            • frozen section can be used to ensure margins are free of tumor
        • radiation
          • 50 Gy pre-operative and 66 Gy postoperative is the standard dose for soft-tissue sarcomas
          • timing of radiation does not impact overall survival
    • Amputation
      • approach
        • level and incision guided by advanced imaging to ensure negative margins
      • technique
        • use of tourniquet without exsanguination
        • maintain meticulous hemostasis
        • meticulous dissection to ensure wide margins, must confirm border free of disease with histology
          • frozen section can be used to ensure margins are free of tumor
    • Surgical resection of lung metastases
      • technique
        • wedge resection
        • lobectomy
  • Complications
    • Radiation induced
      • pre-operative radiotherapy is associated with a > 30% risk for wound complications
      • post-operative radiotherapy is associated with greater radiation-induced morbidity and an increased risk of radiation-induced sarcoma
      • early effects: desquamation, delayed wound healing, infection
      • late effects: fibrosis, post-radiation fracture, possible secondary sarcoma
      • postradiation sarcoma
    • Recurrence
      • local recurrence <10% with radiation and surgery
      • following resection, the most common location for recurrence of a low-grade STS is locally
    • Unplanned excision
      • CT chest, abdomen, pelvis to exclude metastasis
      • MRI of the limb to determine the degree of contamination, post-operative changes, and to assess margins
      • revision surgery
        • limb salvage + radiation therapy
        • amputation
      • unplanned excision is associated with higher plastic reconstruction of the affected region
  • Prognosis
    • Natural history of disease
      • often slow-growing painless mass
      • metastasis is most commonly to the lung
      • 5% lymph node metastasis (most commonly epithelioid, synovial, angiosarcoma, rhabdomyosarcoma, clear cell)
    • Prognostic variables
      • tumor stage is most important prognostic factor
      • poor prognostic factors include
        • high grade
        • metastatic disease
        • size > 5 cm
        • tumor location below the deep fascia
        • delay in diagnosis
        • unplanned excision
          • most common error in STS treatment
          • referral to orthopaedic oncologist for multidisciplinary workup and repeat excision is critical
    • Survival with treatment
      • <10% local recurrence with surgery and radiation
      • mortality up to 50% with high-grade disease
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